Osteopathia striata is an unusual skeletal condition that can affect the bones of the skull quite markedly and result in dental problems that have rarely been discussed in the literature. The characteristic features exhibited by the patient in this case report were dense maxillary and mandibular bone, a high forehead with frontal bossing, a broad nasal bridge, prominent zygomatic arches and thickened angles of the mandible. The mouth opening was limited as a result of sclerosis of the mastoid process, and right lateral excursion of the mandible was reduced, probably because of a deformity of the left condyle.